Papillon-Lefevre Syndrome: A Rare Case Report in Jazan, Kingdom of Saudi Arabia
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Annals of R.S.C.B., ISSN:1583-6258, Vol. 25, Issue 4, 2021, Pages. 11471 - 11476
Received 05 March 2021; Accepted 01 April 2021.
Papillon-Lefevre Syndrome: A Rare Case Report in Jazan, Kingdom of
Saudi Arabia
Running title: Papillon-Lefevre Syndrome
Authors
1. Dr. Naveen Reddy
Assistant Professor, Department of Prosthodontics
College of Dentistry, Jazan University, Kingdom of Saudi Arabia
2. Dr. Hafiz Adawi
Assistant Professor, Department of Prosthodontics
College of Dentistry, Jazan University, Kingdom of Saudi Arabia
3. Dr.WaelIbraheem
Assistant Professor, Department of Periodontics
College of Dentistry, Jazan University, Kingdom of Saudi Arabia
4. Hamza Khalid Allauddin Khan
6th Year Student
College of Dentistry, Jazan University, Kingdom of Saudi Arabia
5. Essa A Adawi
Faculty of Medicine, Jazan University
Kingdom of Saudi Arabia
6. Tasneem Mohammed Ali Jali
Dental Practitioner, Kingdom of Saudi Arabia
Corresponding author
Dr. Naveen Reddy
Assistant Professor, Department of Prosthodontics
College of Dentistry
Jazan University, Kingdom of Saudi Arabia
Abstract
The PapillonLefevre syndrome is an autosomal recessive conditiondescribed
by several dermatological manifestations andoral conditions such as periodontitis. Its
aetiopathogenesis is thought to be secondary to the mutation of the Cathepsin C gene.
It presents with dermatologic feature of Palmoplantar keratosis which ranges from a
mild psoriasiform scaly skin to overt hyperkeratosis and also affect elbows and knees
This case report presents with an oral manifestations of PapillonLefevre
syndrome in a young boy with missing several teeth, which was rehabilated with
removable partial denture.
Keywords :Papillon-Lefevre Syndrome, partial denture, periodontitis, partial denture
Introduction
In 1924,Papillon and Lefevre first, described PapillonLefevre syndrome (PLS)
[1]. Itis a rare autosomal recessive condition [2, 3].It clinicallypresents
withpalmoplantar hyperkeratosis with a rapidly progressive periodontal disease
resulting intopremature exfoliation of both primary and permanent teeth [1].PLS
disorder can be hereditary, acquired, or relatedwith other syndrome [3].
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Annals of R.S.C.B., ISSN:1583-6258, Vol. 25, Issue 4, 2021, Pages. 11471 - 11476
Received 05 March 2021; Accepted 01 April 2021.
The incidence of the Papillonlefevre syndrome is ranges from 1 to 4 per
million, without genderpreference. A genetic tendency with a larger frequency of
existence in offsprings with consanguineous marriage history. It usually demonstrates
during the first 4 to 5 years of age, affecting the both the deciduous dentition with
anearly periodontal involvement, resulting in to an early loss of teeth. It presents with
dermatologic feature of Palmoplantar keratosis which ranges from a mild psoriasiform
scaly skin to overt hyperkeratosis and also affect elbows and knees [1,2].
This case report presents with young boy with the PapillonLefevre syndrome
with missing several teeth, and he was managed with anterior removable partial
denture prosthesis
.
Case Report
A10-year-old boy by reported to Comprehensive Care Clinics, College of
Dentistry, Jazan University with complaints of loose teeth and discomfort
in chewing along with recurrently swollen and friable gums, Panaromic
radiograph confirms with missing several teeth (Fig 1, 2). Patient also
complained of persistent thickening, flaking and scaling of the skin of
palms and soles (Fig 3, 4). He had premature shedding of deciduous
teeth. His family history was non-contributory to his conditions suchas yellowish,
keratotic, confluent plaques which affected the skin of his palms and soles. He was
diagnosed a case of PapillonLefevre syndrome.The patient was managed with
extraction of teeth11 21 12 22 which were periodontal involved with grade III
mobility, following it alginate impressions were made to prepare Interim Removable
Prosthesis (RPD) (Fig 5). Patient was kept under regular follow-up after delivering
the RPD.
Discussion
The aetiopathogenesisforPapillonlefevre syndrome is thought to be due to
microbiologic, immunologic, and genetic causes by several authors. The causative
organisms is said to be due to Porphyromonasgingivalis, Actinobacil-
lusactinomycetemcomitans, Treponema denticolaand Fusobacteriumnucleatum. It is
also stated that neutrophil dysfunction in the form of decreased chemotactic and
phagocytic activities could be linked with this condition. Currentresearch have
advocated that the inactivation of the cathepsin C gene was liable for the skin and the
dental abnormalities of this syndrome. Henceforth, the treatment inpatients with this
syndrome is vquitedifficult [1,2, 3].
This syndrome is characterized by hyperkeratosis of the elbow and the knee,
palmoplantar hyperkeratosis with severe early onset periodontitis, and premature loss
of the both the dentition and [1]. In occasional cases,upper portions of hands and feet,
eyelids, and other parts ofthe body may be invloved [3]. Patients generally presents
with hypodontia and periodontitis [3].
In the present case, the patient’s dermatological history intensely suggested it
as a case ofPapillonLefevre syndrome. The laboratory findings of the blood picture
and the liver function tests were within normal limit.
The differential diagnosis for this condition consist of
hypophosphatemia,acrodynia, and cyclic neutropaenia. It differs from acrodynia or
mercury poisoning by the presence of muscle pain, erythocyanosis,tachycardia,
psychic disturbances, insomnia, and dystrophic enamel. The clinical features of
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Annals of R.S.C.B., ISSN:1583-6258, Vol. 25, Issue 4, 2021, Pages. 11471 - 11476
Received 05 March 2021; Accepted 01 April 2021.
bowing of the femur and the tibia,knock-knee, hypoplastic teeth,enlarged wrists, and
increased amounts of phosphoethanolamine in the urine differentiates
hypophosphatasia from this syndrome. In cyclic neutropaenia, the palmoplantar
hyperkeratosis is absent [1,2,3]. It presents with aggressive periodontitis and
radiographically, it looks as floating teeth in soft tissue [4]. The related featuresalso
comprise of intracranialcalcifications, susceptibility to bacterial infections, and mental
retardationbecause of the rapid alveolar bone resorption [5]. Mutations in the CTSC
gene havebeen reported to result in PLS and the complete absenceof cathepsin C
activity is required in order to developthe clinical phenotype of PLS [6, 7].
The management of this condition should compriseof a team of dermatologists
and dentist. Oral retinoids likeetretinate, acitretin, and isotretinoin are used to treat
both the cutaneous and the dental defects in the Papillon-Lefevre syndrome [1]. The
dental management consists of scaling and root planning, professional prophylaxis,
basic instructions on oral hygiene and use of 0.12% Chlorhexidine mouth rinse,
periodontal debridement with amoxicillin capsules (250 mg) and metronidazole
tablets (200 mg) to control infection and prosthodntic rehabilitation for missing teeth
[7].
Reported articles hasshown that, for the patients with the PapillonLefevre
syndrome, some modifications are needed during the construction of the partial or
complete denture prosthesis. The various modifications such as; keeping the vertical
dimension of the occlusion low, use of selective pressure impressions, keeping the
vertical dimension of the occlusion low and the use of a monoplane and the narrow
posterior teeth and a hollow maxillary denture for the upper arch [1]. The
prosthodontic management in general, should include the preservation of the alveolar
ridge, preferably by using an implant prosthesis [1]. The rehabilitation can be done
using implants, a modified conventional complete denture with modifications, such as
thea low vertical dimension at the occlusion, use of the selective pressure impression
technique, narrow posterior teeth, a balanced occlusion and a hollowmaxillary denture
by using a silicone putty as a weight reduction approach [1, 8, 9]. The carious teeth
were restored and hopeless teeth with severe bone loss were extracted.
A patient can be recalled at 3 monthly intervals to evaluate ridge resorptionand
patient eating habit.Thiscase report can guide management of patients
withPapillonLefevre.
Conclusion
The Papillonlefevre syndrome hapers eating habit, aesthetic, along with social
and psychological wellbeing at a very young age. Thus, an oral rehabilitation with a
complete or a partial denture is necessary, with a future consideration for an implant
supported prosthesis.
References
1. Sreeramulu B, Haragopal S, Shalini K, SudhaMadhuri D, Kiran G. The
Prosthodontic Management of a Young Edentulous Patient with the
PapillonLefevre Syndrome- A Rare Case Report. Journal of Clinical and
Diagnostic Research. 2012 December, Vol-6(10): 1808-1811
2. Yousry YM, EL-Latif AEA and El-Gawad RYA. Case Report: Clinical
manifestation and dental management of Papillon-Lefèvre syndrome [version 1;
peer review: 2 approved] F1000Research 2018, 7:1420
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Annals of R.S.C.B., ISSN:1583-6258, Vol. 25, Issue 4, 2021, Pages. 11471 - 11476
Received 05 March 2021; Accepted 01 April 2021.
3. Chedid JC,Salameh M,El-Outa A, Ziad E. F. Noujeim. Papillon-Lefèvre
Syndrome: Diagnosis, Dental Management, and a Case Report. Case Reports in
Dentistry. Volume 2019, Article ID 4210347, 7 pages
4. de Andrade MG, FragaFernandes AC, de Souza Dantas BPS, Ramos Silva LO, de
Carvalho MMM, et al. (2018) Papillon-Lefèvre Syndrome - A Rare Case Report.
JSM Dent 6(2): 1109.
5. Fageeh HN.Papillon–Lefèvre Syndrome: A Rare Case Report of Two Brothers
and Review of the Literature. International Journal of Clinical Pediatric Dentistry,
July-August 2018;11(4):352-355
6. AIBarrak ZM, Alqarni AS, Elna P. Chalisserry3 and Sukumaran Anil2. Papillon–
Lefèvre syndrome: a series of five cases among siblings. AIBarrak et al. Journal
of Medical Case Reports (2016) 10:260
7. Sharma A, Kaur G, Sharma A. Papillon-Lefevre syndrome: A case report of 2
affected siblings. J Indian SocPeriodontol 2013;17:373-7
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report. J Indian SocPedodPrev Dent 2008;26:171-4
9. Yaqoob Khan F, Jan SM, Mushtaq M. Papillon-Lefèvre syndrome: Case report
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(2); 261-265
Legends for illustration
Images
Fig 1: Intra oral view indicating missing lower anterior teeth and malpositioned upper
anteriors
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Annals of R.S.C.B., ISSN:1583-6258, Vol. 25, Issue 4, 2021, Pages. 11471 - 11476
Received 05 March 2021; Accepted 01 April 2021.
Fig 2: radiographic view with OPG indicating missing several teeth and
malpositioned upper anteriors
Fig 3: Dermatologic manifestations in the form of hyperkeratosis of palms
http://annalsofrscb.ro 11475Annals of R.S.C.B., ISSN:1583-6258, Vol. 25, Issue 4, 2021, Pages. 11471 - 11476
Received 05 March 2021; Accepted 01 April 2021.
Fig 4: Dermatologic manifestations in the form of hyperkeratosis of soles
Fig 5: Intra oral view indicating replaced upper and lower anterior teeth with
RPD
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